Brief Article
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World J Gastroenterol. Aug 21, 2013; 19(31): 5131-5137
Published online Aug 21, 2013. doi: 10.3748/wjg.v19.i31.5131
Connective tissue diseases in primary biliary cirrhosis: A population-based cohort study
Li Wang, Feng-Chun Zhang, Hua Chen, Xuan Zhang, Dong Xu, Yong-Zhe Li, Qian Wang, Li-Xia Gao, Yun-Jiao Yang, Fang Kong, Ke Wang
Li Wang, Feng-Chun Zhang, Hua Chen, Xuan Zhang, Dong Xu, Yong-Zhe Li, Qian Wang, Yun-Jiao Yang, Fang Kong, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100005, China
Li-Xia Gao, Department of Rheumatology, Second Hospital of Hebei Medical University, Shijiazhuang 050000, Hebei Province, China
Ke Wang, Institute of Basic Medical Sciences and School of Basic Medicine, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100005, China
Author contributions: Zhang FC designed the study; Wang L, Zhang X, Xu D, Li YZ, Wang Q and Gao LX enrolled the subjects; Wang K recalculated the data; Wang L, Yang YJ and Kong F analyzed the data; Wang L wrote the manuscript; Zhang FC and Chen H revised the manuscript.
Supported by Grants from the Research Special Fund for Public Welfare Industry of Health, No. 201202004; the National Major Scientific and Technological Special Project for “Significant New Drugs Development”, No. 2012ZX09303006-002; and the National High Technology Research and Development Program of China, No. 2011AA020111
Correspondence to: Feng-Chun Zhang, MD, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, No. 41 Da Mu Cang, Western District, Beijing 100032, China. zhangfccra@yahoo.com.cn
Telephone: +86-10-69158792 Fax: +86-10-69158794
Received: April 6, 2013
Revised: June 3, 2013
Accepted: July 18, 2013
Published online: August 21, 2013
Processing time: 134 Days and 23.1 Hours
Abstract

AIM: To establish the frequency and clinical features of connective tissue diseases (CTDs) in a cohort of Chinese patients with primary biliary cirrhosis (PBC).

METHODS: Three-hundred and twenty-two Chinese PBC patients were screened for the presence of CTD, and the systemic involvement was assessed. The differences in clinical features and laboratory findings between PBC patients with and without CTD were documented. The diversity of incidence of CTDs in PBC of different countries and areas was discussed. For the comparison of normally distributed data, Student’s t test was used, while non-parametric test (Wilcoxon test) for the non-normally distributed data and 2 × 2 χ2 or Fisher’s exact tests for the ratio.

RESULTS: One-hundred and fifty (46.6%) PBC patients had one or more CTDs. The most common CTD was Sjögren’s syndrome (SS, 121 cases, 36.2%). There were nine cases of systemic sclerosis (SSc, 2.8%), 12 of systemic lupus erythematosus (SLE, 3.7%), nine of rheumatoid arthritis (RA, 2.8%), and 10 of polymyositis (PM, 3.1%) in this cohort. Compared to patients with PBC only, the PBC + SS patients were more likely to have fever and elevated erythrocyte sedimentation rate (ESR), higher serum immunoglobulin G (IgG) levels and more frequent rheumatoid factor (RF) and interstitial lung disease (ILD) incidences; PBC + SSc patients had higher frequency of ILD; PBC + SLE patients had lower white blood cell (WBC) count, hemoglobin (Hb), platelet count, γ-glutamyl transpeptidase and immunoglobulin M levels, but higher frequency of renal involvement; PBC + RA patients had lower Hb, higher serum IgG, alkaline phosphatase, faster ESR and a higher ratio of RF positivity; PBC + PM patients had higher WBC count and a tendency towards myocardial involvement.

CONCLUSION: Besides the common liver manifestation of PBC, systemic involvement and overlaps with other CTDs are not infrequent in Chinese patients. When overlapping with other CTDs, PBC patients manifested some special clinical and laboratory features which may have effect on the prognosis.

Keywords: Cirrhosis; Biliary; Connective tissue disease; Sjögren’s syndrome; Systemic sclerosis; Raynaud phenomenon

Core tip: This study demonstrated that primary biliary cirrhosis (PBC) is a complicated disease that not only involves the liver but also often coexists with other connective tissue diseases (CTDs). Evaluation of our cohort of 322 Chinese PBC patients showed that Sjögren's syndrome was the CTD that most frequently coexisted with PBC. In addition, it was also shown that when CTDs coexist with PBC, the clinical features and the disease course are different from those in patients with PBC alone. Our collective results suggest that Chinese patients with PBC may benefit from assessment of systemic involvement and screening for CTDs through detection of autoantibodies.